No abstract available.
Primary Myelofibrosis*

No abstract available.
Echocardiography* ; Endocarditis*

Schilder’s disease is a rare sporadic demyelinating disease of the brain. We report a girl with Schilder’s disease who had undergone Kasai operation for biliary atresia. The patient presented with acute right hemiparesis. Brain magnetic resonance imaging (MRI) showed a single large tumefactive white matter lesion. A diagnosis of Schilder’s disease was based on clinical features and MRI findings. The patient showed dramatic clinical improvement and significant regression of the lesion in response to high-dose intravenous methyl prednisone, and remained free of relapse of other neurologic problems during the 3-year follow-up.

Obstructive sleep apnea syndrome (OSAS) is a disorder of breathing during sleep characterized by prolonged partial upper airway obstruction or intermittent complete obstruction that disrupts normal ventilation during sleep and normal sleep patterns. Untreated OSAS can cause various problems such as cognitive deficit, attention deficit hyperactivity disorder, hypertension, cardiovascular disease, failure to thrive and mood disorder. Therefore, early diagnosis and proper management is very important. The gold standard of diagnosis of OSAS is overnight polysomnography. Treatment includes the use of continuous positive airway pressure (CPAP) and weight loss in obese children. Here, we present an obese 15-year-old boy presented with obstructive sleep apnea and attention deficit. He was diagnosed with OSAS by polysomnography and was successfully treated with CPAP.
Adolescent ; Airway Obstruction ; Attention Deficit Disorder with Hyperactivity ; Cardiovascular Diseases ; Child ; Continuous Positive Airway Pressure* ; Diagnosis ; Early Diagnosis ; Failure to Thrive ; Humans ; Hypertension ; Male ; Mood Disorders ; Obesity ; Polysomnography ; Respiration ; Sleep Apnea, Obstructive* ; Ventilation ; Weight Loss

PURPOSE: The purpose of this study was to describe the ultrasonographic features and assess the diagnosticvalue of sonography in the evaluation of children with Henoch-Schonlein purpura. MATERIALS AND METHODS: BetweenOctober 1993, and Febuary 1998, 67 children with Henoch-Schonlein purpura underwent abdominal ultrasonography,which in 13 was used for follow up. Bowel wall thickness and location, pattern of color Doppler signal in thethickened bowel wall, the size and location of enlarged mesenteric lymph node and the presence of ascites wereevaluated. RESULTS: In 42 cases(63%), sonographic findings were positive, and indicated mesentericlymphadenopathy(n=21), small bowel wall thickening(n=20), and ascites(n=17). Thickened bowels were demonstrated atthe ileum in 11 cases, the jejunum in five, the duodenum in one, and combined wall thickening at the duodenum andjejunum in two ; thickening of the duodenum and ileum was seen in one case. Thickness varied from 3 to 10mm(mean :6.5 mm). On follow-up sonography, regression of bowel wall thickening was observed earlier than that of mesentericlymphadenopathy or ascites, and correlated well with improved abdominal symptoms. CONCLUSION: Abdominalultrasonographic manifestations of Henoch-Schonlein purpura were bowel wall thickening, mesenteric lymphadenopathyand ascites. Sonography was a simple and useful method for the evaluation of gastrointestinal manifestation ofHenoch-Schonlein purpura.
Ascites ; Child ; Duodenum ; Follow-Up Studies ; Gastrointestinal Tract ; Hemorrhage ; Humans ; Ileum ; Jejunum ; Lymph Nodes ; Purpura ; Purpura, Schoenlein-Henoch* ; Ultrasonography

No abstract available.

A case of metastatic alveolar soft part sarcoma is presented with clincal, pathological and radiological features. Alveolar soft part sarcoma is a rare soft-tissue neoplasm that is malignant and invariably fatal. It was first described and named by Christopherson, et al. in 1952. Since 1952 numerous examples of this tumor have been reported and have been studied with the electron microscope, but there is still considerable uncertainty as to the exact histogenesis of the tumor. Most cases occur in young females involving the lower extrimities especially in the right side. The most initial presenting symptom is a slowgrowing painless mass and the principal metastatic sites are the lungs, followed by the brain and skeleton. Cerebral metastasis, in fact, may be the first manifestation of the disease. Treatment is radical surgical excision but radiotherapy and chemotherapy are less effective. We present the reported case of metastatic alveolar soft pat sarcoma with electron microscopic findings.
Brain ; Drug Therapy ; Female ; Humans ; Lung ; Neoplasm Metastasis ; Radiotherapy ; Sarcoma ; Sarcoma, Alveolar Soft Part* ; Skeleton ; Uncertainty

No abstract available.
Hemorrhoidectomy*

Recent genetic advances allow for identification of the genetic etiologies of epilepsy within individual patients earlier and more frequently than ever. Specific targeted treatments have emerged from improvements in understanding of the underlying epileptogenic pathophysiology. These targeted treatment strategies include modifications of ion channels or other cellular receptors and their function, mechanistic target of rapamycin signaling pathways, and substitutive therapies in hereditary metabolic epilepsies. In this review, we explore targeted treatments based on underlying pathophysiologic mechanisms in specific genetic epilepsies.

Recent genetic advances allow for identification of the genetic etiologies of epilepsy within individual patients earlier and more frequently than ever. Specific targeted treatments have emerged from improvements in understanding of the underlying epileptogenic pathophysiology. These targeted treatment strategies include modifications of ion channels or other cellular receptors and their function, mechanistic target of rapamycin signaling pathways, and substitutive therapies in hereditary metabolic epilepsies. In this review, we explore targeted treatments based on underlying pathophysiologic mechanisms in specific genetic epilepsies.